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Published on: August 11, 2018
Lymphocyte blastogenic responses in sickle cell disease
S Taylor1, S Shacks, S Villicana
1Department of Pediatrics, Charles R. Drew University of Medicine and Science, King/Drew Medical Center, Los Angeles, California 90059.
Insights
Sickle cell disease (SCD) patients in crisis show significantly impaired cell-mediated immunity, with reduced lymphocyte blastogenic responses to mitogens like phytohemagglutinin (PHA) and antigens. This immune dysfunction is particularly pronounced during crisis, especially when infection is present.
Area of Science:
- Immunology
- Hematology
Background:
- Cell-mediated immunity (CMI) in sickle cell disease (SCD) remains under-investigated.
- Understanding immune responses in SCD is crucial for managing complications.
Purpose of the Study:
- To assess lymphocyte blastogenic responses in SCD patients during steady state and crisis.
- To compare immune function in SCD patients with healthy controls and infected individuals.
Main Methods:
- Evaluated lymphocyte blastogenic responses using phytohemagglutinin (PHA) and antigens (Candida albicans, Tetanus Toxoid).
- Studied 62 SCD patients (steady state and crisis), 30 healthy controls, and 10 infected controls.
- Assessed responses via stimulation index and mean counts per minute.
Main Results:
- 86% of steady-state SCD patients and 100% of healthy controls showed normal responses.
- Only 20% of SCD crisis patients exhibited normal blastogenic responses.
- SCD crisis patients displayed significantly depressed proliferation to PHA (70%) and antigens (55% to Candida, 30% to Tetanus).
Conclusions:
- The crisis state in SCD profoundly impairs blastogenic responses, particularly to PHA.
- In vitro antigenic stimulation is also affected, though to a lesser extent than mitogen response.
- Infection exacerbates immune dysfunction in SCD crisis patients.
Abstract:
Cell mediated immunity (CMI) has not been extensively studied in sickle cell disease (SCD). In this study, an assessment of lymphocyte blastogenic responses was done in 62 SCD patients (62 steady state and 25 in crisis with or without documented infection), along with 30 normal controls in good health and 10 with various infections. The mitogen phytohemagglutinin (PHA) and two antigens (Candida albicans and Tetanus Toxoid) were used as stimuli. An evaluation of these results by stimulation index revealed 53/62 (86%) of SCD steady state patients, 30/30 (100%) of healthy controls and 7/10 (70%) of normal control patients with infection positively responded to all three stimuli. Conversely, only 5/25 (20%) of SCD crisis state patients had totally normal blastogenic responses. Analysis of the data from SCD crisis patients revealed 14/20 (70%) with abnormally depressed proliferation to PHA, 11/20 (55%) to Candida and 6/20 (30%) to Tetanus. When comparisons were made by mean counts per minute, the most striking finding was the PHA response in the SCD crisis state. In this group the mean counts per minute were significantly less than the three other groups by statistical analysis. This data suggests that the crisis state, especially when associated with infection, profoundly effects blastogenic responses to PHA and to a lesser degree in vitro antigenic stimulation. The normal group with infection showed similar diminished blastogenesis to antigen, but no effect on mitogen response.
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